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Schilder disease (encephalitis periaxialis diffusa)


Schilder Disease (Encephalitis Periaxialis Diffusa)

General: Lesions situated in the su 555e49f bcortical white matter (area 17); occurs in males; any age; etiology unknown; possibly toxic, infectious, or abiotrophic neural defects.

Ocular: Nystagmus; extraocular muscle palsy, either nuclear or supranuclear; hemianopsia (in occipital lobe involvement); optic nerve, chiasm, and tract involvement can lead to blindness; papilledema; optic neuritis; optic atrophy; central scotomata.

Clinical: Progressive spastic paralysis; progressive mental deterioration; irritability and peevishness; deafness if the temporal lobe becomes involved; tremor; dullness; characteristically bilateral lesions in the brain.



Francois J. Ocular manifestations in demyelinating disease. Adv Ophthalmol 1979; 39:1-36.

Martin JJ, Guazzi GC. Schilder's diffuse sclerosis. Dev Neurosci 1991; 13:267-273.

Powers JM, Schaumburg HH. Adrenoleukodystrophy (sex-linked Schilder's disease): a pathogenetic hypophysis based on ultrastructural changes in adrenal cortex, peripheral nerve and testis. Am J Pathol

Schilder P. Zur Kenntnis der Sogenannten Diffusen Sklerose (Ueber Encephalitis Periaxialis Diffusa). Z Ges Neurol Psychiatr




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